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Half of the patients with anti-MDA5 antibodies received more than three medications including intravenous cyclophosphamide, especially patients with ILD. Patients with anti-MDA5 antibodies were more likely to achieve drug-free remission (29% vs 21%) and less likely to relapse (26% vs 44%) than others. Anti-MDA5 antibodies are the most common MSA type in Japan, and patients with this antibody are characterized by ILD at onset, multiple medications including intravenous cyclophosphamide, drug-free remission, and a lower frequency of relaps